• Journal of Yeungnam Medical Science
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• v.39 no.1
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• pp.3-11
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• 2022

Lymphoma is the most common primary tumor of the orbit, accounting for 55% of all orbital malignancies. When divided into histopathological subtypes, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises the largest proportion. Clinical manifestations are unspecific, but in patients with slow-growing painless orbital mass, or red conjunctival lesion suggestive of 'salmon patch', ocular adnexa lymphoma (OAL) should be suspected. Although the pathogenetic mechanism of ocular adnexal MALT lymphoma (OAML) is not yet fully understood, the relationship between OAML and Chlamydia psittaci has been hypothesized recently, similar to that between gastric MALT lymphoma and Helicobacter pylori. This suggests a new treatment option for OAML; bacterial eradication therapy with systemic antibiotics. Several other treatment methods for OAML have been introduced, but no treatment guidelines have been established yet. In this article, we summarize the current knowledge on the clinical features, pathogenesis, diagnostic methods, therapeutic strategies, and prognosis of OAML.

• BLOOD RESEARCH
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• v.53 no.4
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• pp.307-313
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• 2018

Background Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand. Methods We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes. Results Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22-86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01). Conclusion We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.8
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• pp.3727-3731
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• 2016

Background: The most common type of ocular lymphoma is non-Hodgkin lymphoma (NHL), categorized into two groups: indolent (slow growing) and aggressive (rapid growing). Differentiating benign reactive lymphoid hyperplasia (RLH) from malignant ocular adnexal lymphoma (OAL) is challenging. Histopathology, immunohistochemistry (IHC) and flow cytometry have been used as diagnostic tools in such cases. Materials and Methods: In this retrospective case series, from 2002 to 2013 at Farabi Eye Center, 110 patients with ocular lymphoproliferative disease were enrolled. Prevalence, anatomical locations, mean age at diagnosis and the final diagnosis of the disease with IHC were assessed. Comparison between previous pathologic diagnoses and results of IHC was made. Immunoglobulin light chains and B-cell and T-cell markers and other immuno-phenotyping markers including CD20, CD3, CD5, CD23, CD10, CYCLIND1 and BCL2 were evaluated to determine the most accurate diagnosis. The lymphomas were categorized based on revised European-American lymphoma (REAL) classification. Results: Mean age ${\pm}$ SD (years) of the patients was $55.6{\pm}19.3$ and 61% were male. Patients with follicular lymphoma, large B-cell lymphoma or chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL) tended to be older. Nine patients with previous diagnoses of low grade B-cell lymphoma were re-evaluated by IHC and the new diagnoses were as follows: extranodal marginal zone lymphoma(EMZL) (n=1), SLL(n=1), mantle cell lymphoma (MCL) (n=3), reactive lymphoid hyperplasia RLH (n=2). Two cases were excluded due to poor blocks. Flow cytometry reports in these seven patients revealed SLL with positive CD5 and CD23, MCLwith positive CD5 and CyclinD1 and negative CD23, EMZL with negative CD5,CD23 and CD10. One RLH patient was negative for Kappa/Lambda and positive for CD3 and CD20 and the other was positive for all of the light chains, CD3 and CD20. Orbit (49.1%), conjunctiva (16.1%) and lacrimal glands (16.1%) were the most common sites of involvement. Conclusions: Accurate pathological classification of lesions is crucial to determine proper therapeutic approaches. This can be achieved through precise histologic and IHC analyses by expert pathologists.

• Radiation Oncology Journal
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• v.35 no.3
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• pp.249-256
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• 2017

Purpose: We retrospectively reviewed the results of radiotherapy for localized ocular adnexal MALT lymphoma (OAML) to investigate the risk factors of cataract. Methods: Sixty-seven patients with stage IE OAML treated with radiotherapy at Seoul St. Mary's Hospital from 2001 to 2016 were included. Median treatment dose was 30 Gy. Lens protection was done in 52 (76%) patients. Radiation therapy (RT) extent was as follows: superficial (82.1%), tumor mass (4.5%), and entire orbital socket (13.4%). The risk factors for symptomatic cataract were analyzed using the Cox proportional hazard model. Results: Median follow-up time was 50.9 months (range, 1.9 to 149.4 months). All patients were alive at the time of analysis. There were 7 recurrences and there was no local recurrence. Median time to recurrence was 40.4 months. There were 14 cases of symptomatic cataract. Dose >30 Gy had hazard ratio of 3.47 for cataract (p = 0.026). Omitting lens protection showed hazard ratio of 4.10 (p = 0.008). Conclusions: RT achieves excellent local control of ocular MALT lymphoma. Consideration of RT-related factors such as lens protection and radiation dose at the stage of RT planning may reduce the risk of RT-induced cataract after radiotherapy.

• The Journal of the Korea Contents Association
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• v.7 no.12
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• pp.139-144
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• 2007

Radiotherapy which is the most effective for orbit lymphoma has been used increasingly due to the increase of orbit or ocular adnexal tumor patients. Curative effects and convalescence have been being more satisfied thanks to remarkable development of cancer chemotherapy and medical treatments, but side effects such as cataract, dry eye and retinopathy still break out. Thus, in this study, a Lens Shielding Device (LSD hereafter) was designed to prevent occurring of cataract due to radiation therapy for orbit lymphoma and its efficacy through dosimetry were evaluated. And in this paper, its manufacturing process was also explained. LSD is composed of a cover body covering the lens and a side fixing part supporting the cover body. To measure radiation, the patient therapy conditions were simulated and the measurement of the radiation was conducted with Thermo Luminescence Detector (TLD) and Markus chamber. The average TLD value was 5.7% and the TLD value and Markus chamber value were acquired as 4.2% and 5.1% respectively at 6 mm depth where zero lens center was located. Only 1.5Gy ($300Gy{\times}\;5%$) or 5% of total 30Gy with 9 MeV electron beam is estimated to affect on patient's lens. That is smaller dose than the threshold value of cataract (2GY) or the value (5Gy) that was reported to cause cataract in clinical conditions. Thus, these findings suggest that LSD be very useful for prevention of cataract during radiotherapy for malignant lymphoma of orbit and ocular adnexa. Furthermore, it might be possible to reduce patient's discomfort caused by alien substances and to make it easier to fix the device with customized manufacturing manners.